Contemporary challenges in the diagnosis and management of chronic inflammatory demyelinating polyneuropathy

ABSTRACT

Introduction

Despite extensive research, multiple inter-related diagnostic and management challenges remain for chronic inflammatory demyelinating polyneuropathy (CIDP).

Areas Covered

A literature review was performed on diagnosis and treatment in CIDP. The clinical features and disease course were evaluated. Investigative techniques, including electrophysiology, cerebrospinal fluid examination, neuropathology, imaging, and neuroimmunology, were considered in relation to technical aspects, sensitivity, specificity, availability, and cost. Available evidenced-based treatments and those with possible efficacy despite lack of evidence, were considered, as well as current methods for evaluation of treatment effects.

Expert Opinion

CIDP remains a clinical diagnosis, supported first and foremost by electrophysiology. Other investigative techniques have limited impact. Most patients with CIDP respond to available first-line treatments and immunosuppression may be efficacious in those who do not. Consideration of the natural history and of the high reported remission rate, of under-recognized associated disabling features, of treatment administration modalities and assessment methods, require enhanced attention.

KEYWORDS:

• Chronic inflammatory demyelinating polyneuropathy
• diagnosis
• management

Article highlights

• CIDP comprises a heterogeneous group of clinical phenotypes.

• Updated EAN/PNS guidelines have separated ‘typical’ forms from ‘variant’ forms, and categorised 2 diagnostic certainty levels, but have excluded paranodopathies and chronic immune sensory polyradiculopathy, from the CIDP spectrum.

• The diagnosis of CIDP remains in practice today, clinical, supported by electrophysiology, which is of high sensitivity and specificity.

• Additional investigations including cerebrospinal fluid examination, neuropathology, imaging and neuroimmunology, have limited diagnostic value in clinical practice for most patients.

• Available first-line treatments for CIDP are effective for most patients with high remission rates reported and immunosuppressants may be effective in refractory cases.

• Enhanced knowledge on natural history, long-term treatment outcomes and remission rates, better evaluation of treatment effects and improved treatment administration modalities are desirable in future.

Declaration of interests

YA Rajabally has received speaker/consultancy honoraria from LFB and Polyneuron, has received educational sponsorships from LFB and CSL Behring and has obtained research grants from LFB and CSL Behring. The author has no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or conflict with the subject matter or materials discussed in this manuscript apart from those disclosed.

Reviewer disclosures

Peer reviewers on this manuscript have no relevant financial or other relationships to disclose.

Additional information

Funding

This paper was not funded.